Autonomic attack (small fiber atrophy) with CIDP-any suggestions?

[KEDASO]

My current diagnosis:

C.I.D.P., (Chronic Inflammatory Demylenating Polyneuropathy), with small fiber atrophy, (autonomic neuropathy) and with Horner’s Syndrome; Benign autoimmune thyroid disease.

My major problem:

I have been told by 3 doctors on my team, that I am dieing of this disease.  Or, rather, will be dieing of the complications of this disease, C.I.D.P.

I am currently on a regimen of plasmaphereses monthly for 5 days straight, (I have been getting it for 5 months now).  The neurologist is trying to regulate the treatments.

The pulmonologist told me to expect to be on a ventilator by next year.  I use a Bi-Pap at home when it is hard to breathe, and when a bad flair comes, I must be rushed to the hospital.

My G.P. now has me on Marinol, (the pill form of marijuana, as it is legal for dispensation of marijuana in California for medical purposes).  It does seem to help the severe nausea, though only stimulated my appetite slightly; though I am in the middle of trying to regulate the Marinol increasing to where it will benefit my body the most.

The main reason I am writing this:  the plasmaphereses helps the motor & seral, but not the attack on the autonomic system. It has hit the nerves controlling the heart, diaphragm, bowels, bladder, stomach, now difficulty to swallow and losing my voice at end of day due to poor exs(ins)pirations. Also, my Ophthmal. is watching in one eye, (the one with the Horner's Syndrome), for optic neuropathy. Any suggestions to halt or slow the attck on the autonomic system? Thanks, Kedaso/Oceanside,CA

[CCF-Neuro-M.D.-SH]

First of all, keep in mind that I am unable to diagnose you because I am unable to examine you, this forum is for educational purposes.

   Autonomic neuropathy is often hard to combat and is generally approached in a symptomatic fashion (we have no cure or way to reverse the damage).  Autonomic symptoms include orthostatic hypotension (pass out/get light headed with standing) and can be treated with midodrine to raise the blood pressure and mestinon to increase autonomic tone.  Autonomic dysfunction also leads to gastric immobility and this is often helped with small frequent meals and medications such as reglan. Some people eventually require a stomach tube if swallowing difficulty continues.  Bladder emptying can also be affected by autonomic problems and may require a implanted stimulator, or superpubic cath if severe.  I would recommend you get urodynamic studies and a urology consult for this question.  The symptoms you describe are more severe than I would suspect for CIDP with small fiber neuropathy, I would suggest a repeat EMG and consulation with a neuromuscular specialist to help make certain your diagnosis is correct.

I hope this has been helpful.

[KEDASO]

Note: Here are my meds now, and as the attack on the autonomic system progresses through my body, more meds keep getting added on.  Below, was a response to a doctor on a different site, re-my medications I have so far.  He had remarked on each med I was on, and I sent back a resonse to him:

Okay-I saw my neurologist yesterday. He does see the plasmaphereses helping the motor nerves, in that roller coaster ride of climbing up to the high point 2 to 3 weeks after the plasmapherese last dose, then come down to the way it was the last week before the next treatment. So, he is going to continue this routine, of 3 days/1day off/2 days. And continue monthly. He told me that even though there is the roller coaster ride of the high where I can walk with a "limp type of short walk, (still no long distance), down to walking with the walker and/or in the wheelchair, that each time it is given, I might be getting a "smidgin" better each time. I guess I will have to wait and see.

But, as far as it hitting the autonomic system, he said no treatment for this esists yet. However, he said he has been hearing of some good responsives with a drug, (he said the name and called it in to my Pharmacist, and I didn't write it down what the name was as I was going to see the name once I was supposed to pick it up from the Pharmacy), that is used my patients after a kidney transplant. So, he prescribed it for me, however, it bounced back from the pharmacy, because they said it was a certain class of drug, and must be approved by my health insurance. The problem there, my pharmacist told me, was that if this is considered experimental for my disease, of which it has, then my health insurance will probably decline it...I have been wiped out by the non-insurance-covered co-pays for all the treatments and Dx trail, that if they won't cover it, neither can I. But, my doctor, who has over 20 CIDP patients, my case being the worse, thinks it my put a kink in the progression of the CIDP through the autonomic system.

As to my drugs, I know exactly what each is for, and will tell you why each was prescribed--on one of them, it is being used experimentally, with very good success:

"Now to your drugs.

Atenolol lowers BP and reduces heart rate. (Last year, the CIDP hit the nerves controlling my heart. Very often, the pulse rate was between 120-130, with a low of 100. This drug brought it down.)

Mestinon. Why? This is for myasthenia gravis, it should be used with extreme caution in respiratory distress. Side effects include nausea, constipation or involuntary defaecation, slowing of pulse, muscle weakness, paralysis. (He is trying this out on me. He has two myasthenia gravis patients, and knows this drug well. He says it sends nerve impulses to the muscle. Below, is copied from Mestinon's website, as to exactly what the drug does:

"MESTINON® prevents the breakdown of acetylcholine (pronounced "a cee til coline") by allowing more acetylcholine to accumulate. Acetylcholine is the chemical that sends nerve impulses to the muscle. With more acetylcholine, there is more control of voluntary functions such as eye movements, limited strength, swallowing and breathing.1,3,4" So, he is trying it out on me, due to the shallow breathing and trouble swallowing, due to the small fiber atrophy, (autonomic attack). My worst flair come from high heat with high humidity. Where I live, it is considered temperate, and rarely have the high heat/high humidity mix. Well, this summer, we had a very unusual season, with MUCH heat & humidity, and had a very bad flair that put me in the hospital. I was put on the Mestinon then, and to tell the truth, I don't know if it was the drug or what, but was able to get through the summer without another very bad flair, (flair-yes, but very bad-no), though September and October are usually our warmest months here, so 1 more month to go...)

Urecholine (bethanechol) for urinary retention. contra-indicted in slow pulse rate and low blood pressure. Side effects, nausea, blurred vision, slow pulse and colic, (This, due to a month-long hospital stay last November. when it hit the bladder. I am able to stay catheter-free, if I use this drug, as well as no retention. Without the drug, I have to go back to a catheter every 12 hours.)

Neurontin, an anticonvulsant for neuropathic pain (not other pains) you are on the maximum dose as in the formulary. (It is being used here for two purposes: 1. Had very bad, what felt like electrical shocks in my spinal cord. I never knew when they would be coming, but would jolt me off the mattress. They first put me on the drug then. 2. Instead of the skin always feeling like a very bad sunburn, the CIDP hit my thigh, from the buttock to the knee, with very intense burning that felt like the muscle were buring, even though I am normally numb there. So, they doubled my dose then.

Flexeril (cyclobenzaprine) is a sedative. (Two years ago, I was experiencing severe muscle spasms, which I started out as a PRN, then became a TID as time went on with increasing spasms. Now the spasms only happen when the Senna starts working on the intestines, but subsides in a few hours afterward, so I now only use the Sennacot when I am bound badly.

Indocid (Indomethacin) a non steroidal anti-inflammatory for joint and connective tissue pain. Notoriously causes bleeding peptic